ArticleBritish journal of cancer2020
CD30 and ALK combination therapy has high therapeutic potency in RANBP2-ALK-rearranged epithelioid inflammatory myofibroblastic sarcoma.
Article in British journal of cancer, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.
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Who cites it
18 citing papers in PubMed, 32 citations in OpenAlex.
- Comprehensive Multiplatform Tyrosine Kinase Profiling Reveals Novel Actionable FGFR Aberrations across Sarcomas Affecting the Young.Molecular cancer therapeutics · 2026Article
- [Ensartinib Combined with Radiotherapy for the Treatment of Advanced Primary Pulmonary Epithelioid Inflammatory Myofibroblastic Sarcoma Harboring TPM3-ALK Fusion: A Case Report].Zhongguo fei ai za zhi = Chinese journal of lung cancer · 2025Article
- Primary cutaneous rhabdomyosarcoma with EWSR1/FUS::TFCP2 fusion: four new cases with distinctive morphology, immunophenotypic, and genetic profile.Virchows Archiv : an international journal of pathology · 2025Article
- Durable response with mutation-guidedRare tumors · 2025Article
- Case Report:Frontiers in oncology · 2025Article
- Thoracic epithelioid inflammatory myofibroblastic sarcoma: a rare and aggressive disease with case report and literature review.Discover oncology · 2024Article
- Clinical features, treatment strategies, and prognosis of epithelioid inflammatory myofibroblastic sarcoma in children: a multicenter experience.Translational pediatrics · 2024Article
- Epithelioid inflammatory myofibroblastic sarcoma treated with Alectinib: a case report and literature review.Frontiers in oncology · 2024Article
- Epithelioid inflammatory myofibroblastic sarcoma with exceptionally long response to lorlatinib-a case report.Therapeutic advances in medical oncology · 2024Article
- Inflammatory myofibroblastic tumor from molecular diagnostics to current treatment.Oncology research · 2024Review
- Managing Inflammatory Myofibroblastic Tumor of the Uterus: A Case Report and Comprehensive Review of Pathological and Therapeutic Approaches.The American journal of case reports · 2023Review
- Frequent CD30 Expression in an Emerging Group of Mesenchymal Tumors With NTRK, BRAF, RAF1, or RET Fusions.Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc · 2023Article
- Epithelioid inflammatory myofibroblastic sarcoma: a case report and brief literature review.Frontiers in oncology · 2023Article
- Case report: Epithelioid inflammatory myofibroblastic sarcoma treated with an ALK TKI ensartinib.Frontiers in oncology · 2023Article
- Primary pulmonary epithelioid inflammatory myofibroblastic sarcoma: a rare entity and a literature review.Journal of pathology and translational medicine · 2022Article
- Systems-Level Mapping of Cancer Testis Antigen 1b/a to Sarcoma Pathways Identifies Activated Ran Binding-2 E3 SUMO-Protein Ligase and Transducin-Like Enhancer Protein 1.Frontiers in genetics · 2022Article
- Review
- Molecular Targets for Novel Therapeutics in Pediatric Fusion-Positive Non-CNS Solid Tumors.Frontiers in pharmacology · 2021Review
Corrections and comments
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Authors and funding
15 authors at 6 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundEpithelioid inflammatory myofibroblastic sarcoma (eIMS) is characterised by perinuclear ALK localisation, CD30 expression and early relapse despite crizotinib treatment. We aimed to identify therapies to prevent and/or treat ALK inhibitor resistance.
methodsMalignant ascites, from an eIMS patient at diagnosis and following multiple relapses, were used to generate matched diagnosis and relapse xenografts.
resultsXenografts were validated by confirmation of RANBP2-ALK rearrangement, perinuclear ALK localisation and CD30 expression. Although brentuximab-vedotin (BV) demonstrated single-agent activity, tumours regrew during BV therapy. BV resistance was associated with reduced CD30 expression and induction of ABCB1. BV resistance was reversed in vitro by tariquidar, but combination BV and tariquidar treatment only briefly slowed xenograft growth compared with BV alone. Combining BV with either crizotinib or ceritinib resulted in marked tumour shrinkage in both xenograft models, and resulted in prolonged tumour-free survival in the diagnosis compared with the relapse xenograft.
conclusionsCD30 is a therapeutic target in eIMS. BV efficacy is limited by the rapid emergence of resistance. Prolonged survival with combination ALK and CD30-targeted-therapy in the diagnosis model provides the rationale to trial this combination in eIMS patients at diagnosis. This combination could also be considered for other CD30-positive, ALK-rearranged malignancies.
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